Complement Activation in Autoimmune Bullous Dermatoses: A Comprehensive Review

Autoimmune bullous dermatoses (AIBD) are characterized by circulating autoantibodies that are either directed against epidermal antigens or deposited as immune complexes in the basement membrane zone. The complement system (CS) can be activated by autoantibodies, thereby triggering activation of specific complement pathways. Local complement activation induces a pathogenic inflammatory response that eventually results in the formation of a sub- or intraepidermal blister. Deposition of complement components is routinely used as a diagnostic marker for AIBD. Knowledge from different animal models mimicking AIBD and deposition of complement components in human skin biopsies provides more insight into the role of complement in the pathogenesis of the different AIBD. This review outlines the role of the CS in several AIBD including bullous pemphigoid, epidermolysis bullosa acquisita, mucous membrane pemphigoid, pemphigus, linear IgA-diseases and dermatitis herpetiformis. We also discuss potential therapeutic approaches targeting key complement components, pathways and pathogenic complement-mediated events.
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research

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CONCLUSIONS AND CLINICAL IMPORTANCE: There has been a remarkable expansion of knowledge on Malassezia yeasts and their role in animal disease, particularly since the early 1990's. Malassezia dermatitis in dogs and cats has evolved from a disease of obscurity and controversy on its existence, to now being a routine diagnosis in general veterinary practice. Clinical signs are well recognised and diagnostic approaches are well developed. A range of topical and systemic therapies is known to be effective, especially when predisposing factors are identified and corrected. PMID: 31957204 [PubMed - in process]
Source: Veterinary Dermatology - Category: Veterinary Research Authors: Tags: Vet Dermatol Source Type: research
PMID: 31957203 [PubMed - in process]
Source: Veterinary Dermatology - Category: Veterinary Research Authors: Tags: Vet Dermatol Source Type: research
Publication date: Available online 20 January 2020Source: Integrative Medicine ResearchAuthor(s): Min Hee Kim, Chunhoo Cheon, Hae Jeong Nam, Bonghyun Kim, Inhwa Choi
Source: Integrative Medicine Research - Category: Complementary Medicine Source Type: research
Publication date: Available online 20 January 2020Source: Methods in MicrobiologyAuthor(s): Maria M. Plummer, Charles S. PaviaAbstractImmunoassay detection of specific antigens and host-produced antibodies directed against such antigens constitute two of the most widely used and successful methods for diagnosing infectious diseases and many of the non-infectious autoimmune disorders. We will be reviewing some of the epidemiology, microbiology, and clinical aspects of selected organisms and discuss their diagnosis by describing some of the major antigen detection systems and serologic techniques available to health care pro...
Source: Methods in Microbiology - Category: Microbiology Source Type: research
Since its inception, Kidney International has published seminal articles describing the effects of systemic autoimmune diseases on the kidney. These manuscripts have characterized the pathology and clinical course of these diseases and perhaps most importantly have provided guidance to the nephrology community about how to treat these complex conditions using the best possible evidence available at the time. To illustrate the wealth of information contained within the Kidney International archives, consider the following papers.
Source: Kidney International - Category: Urology & Nephrology Authors: Tags: Editorial: milestones in nephrology Source Type: research
Publication date: Available online 14 December 2019Source: Best Practice &Research Clinical RheumatologyAuthor(s): Ben Mulhearn, Sarah L. Tansley, Neil J. McHughAbstractAutoimmune connective tissue diseases are heterogeneous rheumatic diseases with the potential to affect multiple body systems. Autoantibodies are a characteristic feature of these diseases and are typically highly disease specific. In addition to aiding diagnosis, many autoantibodies have established associations with clinically important disease complications including internal organ involvement. In this chapter, we review the autoantibodies relevant t...
Source: Best Practice and Research Clinical Rheumatology - Category: Rheumatology Source Type: research
Zhou and colleagues detected ectopic lymphoid structures (ELS) that resemble tertiary lymphoid organs in pemphigus vulgaris and pemphigus foliaceous lesions. ELS were characterized by B cells reactive to the desmoglein 3 autoantigen, and were associated with active disease and B cell expansion. B cell differentiation in ELS was supported by the detection of centroblasts, plasmablasts, and plasma cells in these lesions. Chemokines that may induce B cell migration to pemphigus lesions were also detected in ELS.
Source: Journal of Investigative Dermatology - Category: Dermatology Tags: Editorial Source Type: research
Conclusions The clinical trials discussed here, which include several trials investigating novel therapeutic targets, demonstrate that translational research in pemphigus and pemphigoid is a fast-growing field. We thus expect that several novel treatments will be shortly available for the treatment of pemphigus and pemphigoid patients. Given the high, and thus far unmet, medical need in this field (110), this is highly encouraging and will hopefully improve the quality of life of the affected patients. In addition to the compounds and targets described here, several new targets have been recently identified in preclinical...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Autoimmune blistering diseases (AIBD) are a group of chronic diseases affecting the skin and mucous membranes, with different presentation, clinical course, histologic and immunopathologic findings, and different therapeutic approach. Blisters develop as a result of autoantibodies directed against distinct adhesion structures within desmosomes or within the basement membrane zone. The most common AIBD that develops in the elderly is bullous pemphigoid (previously also named “pemphigoid senilis”), but mature patients can also present with other AIBD as mucous membrane pemphigoid, epidermolysis bullosa acquisita,...
Source: Clinics in Dermatology - Category: Dermatology Authors: Source Type: research
This study was conducted to determine the clinicoepidemiologic features of ABDs in elderly patients. Medical records of all ABD patients with disease onset after the age of 60 years who presented to the Autoimmune Bullous Diseases Research Center, Tehran, Iran between April 2003 and March 2013 were reviewed. Patients with dermatitis herpetiformis were not included. During the 10-year period studied, 296 patients with ABD and disease onset after 60 years of age were diagnosed. Bullous pemphigoid (BP) was observed to be the most common ABD (48.3%), followed by pemphigus vulgaris (45.3%), pemphigus foliaceus (3.7%), mucous me...
Source: Skinmed - Category: Dermatology Authors: Tags: Skinmed Source Type: research
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